Clinical Neuroscience

[Report]

LEEL-ŐSSY Lóránt

MARCH 20, 1994

Clinical Neuroscience - 1994;47(03-04)

[Report - International Joint Conference on Stroke and Cerebral Circulation January 7-10, 1994. Bombay, India.]

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Clinical Neuroscience

[Dementia and related problems '93. an overview]

TARISKA Péter

[Based on extensive investigations carried out the epidemiology of dementias in the last decade in Europe the methodology has become unified. The most important elements are: two-step screening in two time periods, population of 4000 or more, unified screening and diagnostic methods. The incidence was 1 p.c. established in the population between the ages of 60–64; the proportion nearly doubled in each 5 year period. Regarding the recently published risk factors of Alzheimer's disease (AD) the possible protective role of smoking and the low educational level seem to be very interesting. In the group of AAMI the biological life events as possible causative factors seem to be of importance. The functional assesment scale of Reisberg is reported from psychometric tests. An outline is given about the clinical diagnostic criteria of AD and vascular dementias based on the widely discussed system published on the latter in 1993. The problems of different clinical subtypes of AD with relevance to biochemical markers are discussed as are the diganostic criteria of Lewy body type dementia. A summary on some new etological results (genetic heterogenety, new possible ways of amyloidogenesis, glutamate-theory, etc.) is given. After highlihgting the importance of the different kinds of psychotherapy and mental training, social factors are stressed, and some ethical conflict situations (driving, coercive measures, etc.) are shortly presented.]

Clinical Neuroscience

[The history of Hungarian neurology (Part I)]

BEKÉNY György

[Every nation has its great people of whom it is proud. In our country, intellectuals think that Hungarians are particularly talented in music and mathematics. However, our outstanding geneticist believes that the genetic background of different abilities is equal between nations and that the different results are caused by external circumstances. Indeed, the "accumulation" of great Hungarian conductors and musicians in the United States, and the careers of many Nobel Prize-winning mathematicians and physicists in the United States, were made possible by the fact that they had to leave their homeland for political reasons. Contrary to what is suggested above, the first 50 years of a very successful and internationally respected period of Hungarian neuroscience were hampered by these circumstances. All the more reason to appreciate the neurological output of this period. I would be glad if the readers of this work would share this opinion. I have selected those publications for illustration which appeared in the so-called 'world languages'. The only exceptions are monographs, theses and one or two major works, some of which have already been published in a foreign language. Due to the limitations of space, many areas of neuroscience could not be discussed. Thus, I have not been able to write about international greats in neuroanatomy such as Mihály Lenhossék, Apáthy, Szentágothai; about neurophysiologists, especially those working on the cochleovestibular system such as Hőgyes, Bárány, Békésy; about neuroendocrinology or about many details of neurochemistry. The importance of neurosurgery, which is closely related to our subject, calls for a separate presentation. A small monograph on the history of Hungarian neuroscience was published in 1976 by István Környey, a great teacher and scholar of Hungarian neurology. In 1992, Zoltán Nagy published a history of Hungarian neurology in the last century under the title Hungarian Neurology in The Last Century. These historical summaries were important precursors to my present work. ]

Clinical Neuroscience

[Voltage mapping studies of generalized spike- wave patterns associated with absence seizures]

CLEMENS Béla

[In this retrospective study, scalp electric fields of ictal generalized spike-wave discharges were analized by the so-called topographic voltage mapping method. The 17 patients displaying absences (with or without other seizures) belonged to different age groups and diverse epileptic syndromes. Main results: 1. Maps derived at the points of the spike and the wave components belong to different classes. A-type spikes show frontal, P-type spikes show posterior voltage maxima. Also atypical spike (map) configurations exist. 2. A spike maps frequently show a characteristic modification along the GSW pattern. 3. Waves can display bilateral (L) or medial (M) frontal voltage maxima. 4. At least in the time window of several weeks, the dominant spike map pattern and the pattern of the waveform was characteristic to each patient. Configurations of the different spike and wave components show some relation to a limited set of clinical data. The combination of A- spikes with L-waves was found in children who had , typical" absence- epilepsies. On the contrary, irregular spectrum of different spikes and M-waves was found in elder absence patients showing rather unfavorable course of their illness.]

Clinical Neuroscience

[Immunological test for idiopathic inflammatory myopathies]

HORVÁTH Rita, ALZIRA A S Carvalmo

[Sceletal muscle biopsy specimens from patients with various inflammatory myopathies – dermatomyositis, polymyositis and inclusion body myositis – have been investigated by immunocytochemical methods with the help of monoclonal antibodies. Conclusions about the pathomechanism of these disorders were saught. In dermatomyositis the humoral immunity and the damage of the small vessels of muscle fibres may play an important role. The appearance of class I MHC antigens on diseased muscle may make the affected tissue a target for cytotoxic T8 cells, and may thus have a role in muscle fibre damage in polymyositis and inclusion body myositis.]

Clinical Neuroscience

[Is there a relationship between CT morphology and the MMS scale achievements in patients with dementia?]

PÉK Márta, BARSI Péter, NAGY Zoltán

[An attempt is made to establish relationships between CT parameters and the achievements on the Mini Mental State (MMS) scale of patients suffering from various types of dementia. The results suggest that the Mini Mental State scores change together in Alzheimer's type of dementias, referring to the global deterioration of functions in contrast to the vascular type of dementias, where the scores on each item change independently of each other. In the combined examination of the two groups the parietal lobe and the volume of the ventricles showed mainly connection with the neuropsychological functions. ]

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Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

A variant of Guillain-Barre syndrome after SARS-CoV-2 vaccination: AMSAN

TUTAR Kaya Nurhan, EYIGÜRBÜZ Tuğba, YILDIRIM Zerrin, KALE Nilufer

Introduction - Coronavirus disease 2019 (COVID-19) is a respiratory infection that has rapidly become a global pandemic and vaccines against SARS-CoV-2 have been developed with great success. In this article, we would like to present a patient who developed Guillain-Barré syndrome (GBS), which is a serious complication after receiving the inactive SARS-CoV-2 vaccine (CoronaVac). Case report – A 76-year-old male patient presented to the emergency department with nine days of progressive limb weakness. Two weeks prior to admission, he received the second dose of CoronaVac vaccine. Motor examination revealed decreased extremity strength with 3/5 in the lower extremities versus 4/5 in the upper extremities. Deep tendon reflexes were absent in all four extremities. Nerve conduction studies showed predominantly reduced amplitude in both motor and sensory nerves, consistent with AMSAN (acute motor and sensory axonal neuropathy). Conclusion - Clinicians should be aware of the neuro­logical complications or other side effects associated with COVID-19 vaccination so that early treatment can be an option.

Clinical Neuroscience

Acute transverse myelitis after inactivated COVID-19 vaccine

ERDEM Şimşek Nazan, DEMIRCI Seden, ÖZEL Tuğba , MAMADOVA Khalida, KARAALI Kamil , ÇELIK Tuğba Havva , USLU Ilgen Ferda, ÖZKAYNAK Sibel Sehür

Vaccines against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) have been rapidly developed to prevent coronavirus disease 2019 (COVID-19) pandemic. There is increasing safety concerns regarding COVID-19 vaccines. We report a 78-year old woman who was presented with tetraparesis, paresthesias of bilateral upper extremities, and urinary retention of one-day duration. Three weeks before these symptoms, she was vaccinated with CoronaVAC vaccine (Sinovac Life Sciences, China). Spine magnetic resonance imaging showed longitudinally extensive transverse myelitis (TM) from the C1 to the T3 spinal cord segment. An extensive diagnostic workup was performed to exclude other possible causes of TM. We suggest that longitudinally extensive TM may be associated with COVID-19 vaccination in this case. To the best of our knowledge, this is the first report of longitudinally extensive TM developing after CoronaVac vaccination. Clinicians should be aware of neurological symptoms after vaccination of COVID-19.

Clinical Neuroscience

Atypical presentation of late-onset Sandhoff disease: a case report

SALAMON András , SZPISJAK László , ZÁDORI Dénes, LÉNÁRT István, MARÓTI Zoltán, KALMÁR Tibor , BRIERLEY M. H. Charlotte, DEEGAN B. Patrick , KLIVÉNYI Péter

Sandhoff disease is a rare type of hereditary (autosomal recessive) GM2-gangliosidosis, which is caused by mutation of the HEXB gene. Disruption of the β subunit of the hexosaminidase (Hex) enzyme affects the function of both the Hex-A and Hex-B isoforms. The severity and the age of onset of the disease (infantile or classic; juvenile; adult) depends on the residual activity of the enzyme. The late-onset form is characterized by diverse symptomatology, comprising motor neuron disease, ataxia, tremor, dystonia, psychiatric symptoms and neuropathy. A 36-year-old female patient has been presenting progressive, symmetrical lower limb weakness for 9 years. Detailed neurological examination revealed mild symmetrical weakness in the hip flexors without the involvement of other muscle groups. The patellar reflex was decreased on both sides. Laboratory tests showed no relevant alteration and routine electroencephalography and brain MRI were normal. Nerve conduction studies and electromyography revealed alterations corresponding to sensory neuropathy. Muscle biopsy demonstrated signs of mild neurogenic lesion. Her younger brother (32-year-old) was observed with similar symptoms. Detailed genetic study detected a known pathogenic missense mutation and a 15,088 base pair long known pathogenic deletion in the HEXB gene (NM_000521.4:c.1417G>A; NM_000521:c.-376-5836_669+1473del; double heterozygous state). Segregation analysis and hexosaminidase enzyme assay of the family further confirmed the diagnosis of late-onset Sandhoff disease. The purpose of this case report is to draw attention to the significance of late-onset Sandhoff disease amongst disorders presenting with proximal predominant symmetric lower limb muscle weakness in adulthood.

Clinical Neuroscience

Autonomic nervous system may be affected after carpal tunnel syndrome surgery: A possible mechanism for persistence of symptoms after surgery

ONDER Burcu, KELES Yavuz Betul

After carpal tunnel surgery, some patients report complaints such as edema, pain, and numbness. Purpose – The aim of this study was to evaluate autonomic nervous system function in patients with a history of carpal tunnel surgery using sympathetic skin response (SSR). Thirty three patients (55 ±10 years old) with a history of unilateral operation for carpal tunnel syndrome were included in the study. The SSR test was performed for both hands. Both upper extremities median and ulnar nerve conduction results were recorded. A reduced amplitude (p=0.006) and delayed latency (p<0.0001) were detected in the SSR test on the operated side compared to contralateral side. There was no correlation between SSR and carpal tunnel syndrome severity. Although complex regional pain syndrome does not develop in patients after carpal tunnel surgery, some of the complaints may be caused by effects on the autonomic nervous system.